Summary

In India, Atlantoaxial dislocation (AAD) is the commonest skeletal craniovertebral junction (CVJ) anomaly, followed by occipitalisation
of atlas and basilar invagination. The usual presentation is progressive neurological deficit (76–95% cases) involving the
high cervical cord, lower brainstem and cranial nerves. The association between vertebro-basilar insufficiency and skeletal
CVJ anomalies is well recognised and angiographic abnormalities of the vertebrobasilar arteries and their branches have been
reported; however, initial presentation of CVJ anomaly as thalamic syndrome due to posterior circulation stroke is extremely
rare. Here, we report one such rare case of thalamic syndrome as the initial presentation of CVJ anomaly with AAD.